Year : 2016 | Volume
: 11 | Issue : 6 | Page : 163--164
International scleroderma symposium: July 16-17, 2016, Bengaluru
Rajkiran Dudam1, Balebail G Dharmanand2,
1 Department of Rheumatology, Star Hospital, Hyderabad, Telangana, India
2 Department of Rheumatology, Sakra World Hospital, Bangalore, Karnataka, India
Balebail G Dharmanand
Department of Rheumatology, Sakra World Hospital, Bangalore, Karnataka
|How to cite this article:|
Dudam R, Dharmanand BG. International scleroderma symposium: July 16-17, 2016, Bengaluru.Indian J Rheumatol 2016;11:163-164
|How to cite this URL:|
Dudam R, Dharmanand BG. International scleroderma symposium: July 16-17, 2016, Bengaluru. Indian J Rheumatol [serial online] 2016 [cited 2020 Jul 13 ];11:163-164
Available from: http://www.indianjrheumatol.com/text.asp?2016/11/6/163/194552
The Indian Rheumatology Association - Karnataka chapter had organized the international scleroderma symposium at Bengaluru this year where various clinical and pathological aspects of this multifaceted disease were discussed.
On day 1, Prof. Maurizio Cutolo started the proceedings with discussion on the pathogenesis of systemic sclerosis (SSc). He spoke about the various genetic and epigenetic mechanisms and stressed on the role of signal transducer and activator of transcription 4 and interferon regulatory factor 5 genetic polymorphisms, microRNAs in the pathogenesis. The role of female sex hormones, stress-triggered neuroendocrine hormones, and the consideration of stress management as a part of treatment were discussed. He spoke on the possible role of circulating progenitor cells and hypoxia as trigger agents.
Later, Prof. Chris Denton spoke on the wholesome management of SSc. He stressed on the importance of early recognition of serious manifestations (interstitial lung disease (ILD), pulmonary artery hypertension (PAH), renal and cardiac involvement) and treating them. He discussed the observations of European Scleroderma Observational Study and the role of stem cell therapy in scleroderma with a special mention on the results of Autologous Stem cell Transplantation International Scleroderma trial.  He discussed the challenging aspects in managing the diffuse subset of SSc patients and the role of current therapies in reducing the mortality.
One of the most challenging issues in SSc is the management of severe Raynaud's and its complications. Prof. Marco Matucci Cerinic discussed the European Scleroderma Trials and Research group (EUSTAR)  recommendations for the treatment of digital vasculopathy in SSc. He stressed on using calcium channel blockers as the first-line agent and to consider intravenous prostanoids in refractory disease. He also spoke on the added efficacy of combining sildenafil with bosentan and the role of bosentan in preventing the development of new ulcers. Role of other agents with anecdotal evidence such as botulinum toxin A, rituximab, digital sympathectomy, and autologous fat grafting was also discussed.
Later, Prof. Chris Denton discussed the risk factors of scleroderma renal crisis (SRC). He spoke about the role of angiotensin-converting enzyme inhibitors in the treatment of SRC and lack of evidence for its use as a prophylactic agent in the prevention of SRC. The role of intravenous vasodilators in SRC, criteria for starting renal replacement therapy, and the usefulness of N-terminal pro-brain natriuretic peptide (NT-pro BNP) as a biomarker were also discussed. He specified the need to wait for 24 months before considering renal transplantation and consideration of alternative pathologies in SSc patients testing positive for systemic lupus erythematosus or antineutrophil cytoplasmic-associated antibody. The rarity of SRC in Indian population was discussed, and Prof. Dinesh Khanna felt the need for more Indian data to compare the phenotypic pattern with the Western population. Later, he spoke on ILD, which presents earlier in comparison to PAH. He stressed on the importance of testing for autoantibodies and the need for obtaining a high-resolution computed tomography chest in all patients with Scl-70 positivity. He mentioned the criteria for starting immunosuppressives in patients with ILD and discussed about scleroderma lung study I and II. ,
He shared his experience of using intravenous pulse cyclophosphamide initially followed by mycophenolate mofetil (MMF) in severe ILD and directly starting with MMF in mild-to-moderate cases. He spoke on the role of rituximab as an add-on therapy in progressive lung disease, the emerging evidence for pirfenidone, and the role of autologous stem cell transplant when other therapies failed.
He discussed the various causes for pulmonary hypertension and the need to look for them, as PAH is not the only cause for pulmonary hypertension in SSc/mixed connective tissue disorder patients. He mentioned about DETECT  algorithm and its use in referring patients for right heart catheterization. He also stressed the need for regular follow-up annually with investigations such as echocardiogram, diffusing capacity of the lungs for carbon monoxide, pulmonary function test, and NT-proBNP levels, as early detection and treatment improves survival.
Prof. Marco discussed the classification of calcinosis cutis and its systemic complications. He discussed the role of topical sodium metabisulfite in calcinosis cutis. He also discussed rituximab, ultrasound-guided needle aspiration, and lavage in refractory cases. Interesting and challenging cases of SSc from India were presented by Dr. S. Nagaraj (Bengalore) and Dr. Kaushik Bhoujani (Mumbai). Dr. Shefali Khannna Sharma (Chandigarh) and Dr. Padmanabha Shenoy (Kochi) presented Indian studies.
Workshops on nail fold capillaroscopy and skin scoring later that day were well useful and well attended.
On day 2, Prof. Dinesh Khanna emphasized the importance of classifying patients into fibrotic or vasculopathic phenotype. He also stressed that all patients need not be started on immunosuppressive therapy. The type and dose of immunosuppressive drug depends on the organ involvement, the duration of symptoms, and the severity. He discussed the role of interleukin-6 (IL-6) in the pathogenesis of SSc and the encouraging results obtained from animal models on blockade of IL-6. He spoke about the FaSScinate trial  and the efficacy of tocilizumab in reducing the skin thickening significantly. Emerging evidence (LOTUS  and Scleroderma Lung Study III [ongoing]) in favor of efficacy, safety, and tolerability of pirfenidone was discussed. Other ongoing trials in ILD including nintedanib (a tyrosine kinase inhibitor) and abituzumab (αv-integrin inhibitor) were also discussed.
The EUSTAR data on the efficacy and safety of rituximab in SSc were shared by Prof. Chris Denton and were pretty encouraging. Prof. Dinesh Khanna gave a short presentation on skin scoring and its use as a surrogate marker for internal organ involvement. He also mentioned about the increased risk of cancer associated with RNA polymerase I antibody and the need to screen for cancer in elderly patients with an explosive onset of scleroderma.
In the end, Prof. Chris Denton discussed the various gastrointestinal manifestations of scleroderma and the role of prokinetics and probiotics in management. He also stressed on the need for adequate nutrition and the role of intravenous immunoglobulin as an immunomodulatory agent in those with established gastrointestinal complications. Dr. Sharath Kumar and Dr. Jacob Mathews from Bengalore presented challenging cases.
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Conflicts of interest
There are no conflicts of interest.
|1||van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, et al. Autologous hematopoietic stem cell transplantation vs. intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: A randomized clinical trial. JAMA 2014;311:2490-8.|
|2||Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009;68:620-8.|
|3||Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006;354:2655-66.|
|4||Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): A randomised controlled, double-blind, parallel group trial. Lancet Respir Med 2016;4:708-19.|
|5||Soukup T, Pudil R, Kubinova K, Hromadkova L, Dusek J, Tosovsky M, et al. Application of the DETECT algorithm for detection of risk of pulmonary arterial hypertension in systemic sclerosis: Data from a Czech tertiary centre. Rheumatology (Oxford) 2016;55:109-14.|
|6||Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME, et al. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): A phase 2, randomised, controlled trial. Lancet 2016;387:2630-40.|
|7||Khanna D, Albera C, Fischer A, Khalidi N, Raghu G, Chung L, et al. An open-label, Phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: The LOTUSS Trial. J Rheumatol 2016;43:1672-9.|